Chronic uveitis occurs in 2 to 34 percent of children with juvenile rheumatoid arthritis. Uveitis can lead to cataracts, glaucoma, band keratopathy, phthisis bulbi, and blindness. Diagnosis of early uveitis is not possible by direct ophthalmoscopy, so a slit-lamp examination must be performed to check for inflammatory cells and increased protein within the anterior chamber of the eye.

There are three major onset types of juvenile rheumatoid arthritis: oligoarticular, polyarticular, and systemic. Children with oligoarticular-onset juvenile rheumatoid arthritis are at the highest risk of developing chronic uveitis and are usually diagnosed between one and five years of age. Chronic uveitis can sometimes be detected at the time of the arthritis diagnosis; if it cannot be detected at diagnosis, it usually will present within the next four to seven years.

The presence of antinuclear antibodies is the serologic marker most strongly associated with chronic uveitis. The antibodies are usually detected in low to moderate titers on HEp-2 cells. Their antigenic specificity is unknown. Immunogenic factors may pre-dispose some children to the development of chronic uveitis; the associated alleles can most often be found in the major histocompatability complex region of chromosome 6 and involve specificities in the HLS-DR, DP, and DQ regions.

The onset of chronic uveitis is usually asymptomatic, which underlines the importance of the slit-lamp examination by an ophthalmologist at the time of diagnosis with arthritis and periodically thereafter. Possible symptoms, though very rare, include redness of the eye, decreased vision, unequal pupils, ocular pain, and headache. Any of these symptoms should prompt an immediate eye examination.

All patients should have a slit-lamp examination within one month of diagnosis with juvenile rheumatoid arthritis. The recommended frequency of ophthalmologic visits for children without confirmed uveitis is given in Table 1. Children with diagnosed uveitis will have their examination schedule determined by a pediatric ophthalmologist.

Frequency of Ophthalmologic Examinations in Patients with Juvenile Rheumatoid Arthritis

Type

Antinuclear bodies

Age at onset (years)

Duration of disease (years)

Risk category

Examination frequency (months)

Oligoarthritis or polyarthritis

Present

≤ 6

≤ 4

High

3

Present

≤ 6

> 4

Moderate

6

Present

≤ 6

> 7

Low

12

Present

> 6

≤ 4

Moderate

6

Present

> 6

> 4

Low

12

Absent

≤ 6

≤ 4

Moderate

6

Absent

≤ 6

> 4

Low

12

Absent

> 6

N/A

Low

12

Systemic disease (e.g., fever, rash)

N/A

N/A

N/A

Low

12

note:Recommendations for follow-up continue throughout childhood and adolescence.

Adapted with permission from Cassidy J, Kivlin J, Lindsley C, Nocton J, for the Section on Rheumatology and the Section on Ophthalmology, American Academy of Pediatrics. Ophthalmologic examinations in children with juvenile rheumatoid arthritis. Pediatrics 2006;117:1844.

Table 1

Frequency of Ophthalmologic Examinations in Patients with Juvenile Rheumatoid Arthritis

Type

Antinuclear bodies

Age at onset (years)

Duration of disease (years)

Risk category

Examination frequency (months)

Oligoarthritis or polyarthritis

Present

≤ 6

≤ 4

High

3

Present

≤ 6

> 4

Moderate

6

Present

≤ 6

> 7

Low

12

Present

> 6

≤ 4

Moderate

6

Present

> 6

> 4

Low

12

Absent

≤ 6

≤ 4

Moderate

6

Absent

≤ 6

> 4

Low

12

Absent

> 6

N/A

Low

12

Systemic disease (e.g., fever, rash)

N/A

N/A

N/A

Low

12

note:Recommendations for follow-up continue throughout childhood and adolescence.

Adapted with permission from Cassidy J, Kivlin J, Lindsley C, Nocton J, for the Section on Rheumatology and the Section on Ophthalmology, American Academy of Pediatrics. Ophthalmologic examinations in children with juvenile rheumatoid arthritis. Pediatrics 2006;117:1844.